Epidermolysis Bullosa Pruriginosa

Abstract

Epidermolysis bullosa (EB) pruriginosa is a rare clinical subtype of dystrophic epidermolysis bullosa (DEB) that is characterized by intense pruritus resulting in hypertrophic, lichenified, prurigo-like plaques and nodules secondary to scratching. The variability in the age of onset, rarity of intact bullae, histologic ambiguities, and close resemblance to other conditions such as acquired inflammatory dermatoses may make diagnosis difficult for this unusual condition, for which fewer than 100 cases have been documented. In this report, we describe 3 cases of EB pruriginosa and review the current literature.

Epidermolysis bullosa pruriginosa: A case report of two first cousins

“…Epidermolysis bullosa pruriginosa is sometimes a difficult diagnosis due to variability in age of onset, rarity of intact bullae, histologic ambiguities and resemblance to acquired inflammatory dermatoses. 3 This variant of dystrophic epidermolysis bullosa may not manifest clinically until adulthood and often misdiagnosed as other acquired skin conditions like nodular prurigo, lichen planus, lichen simplex etc. 4 Mutations in COL7A1 gene encoding the anchoring fibril protein, type VII collagen is linked to Epidermolysis bullosa pruriginosa but genotype-phenotype correlation is not clear.…”

Low-dose naltrexone as treatment for epidermolysis bullosa pruriginosa–associated refractory pruritus

“…Histologic evaluation often reveals hyperkeratosis, acanthosis, a subepidermal blister or cleft, dermal fibrosis, and an associated vascular proliferation, which may be accompanied by a mild dermal chronic inflammatory infiltrate and milia cysts. 4 Direct immunofluorescence is typically negative. 1 Few treatments have proven effective at managing symptoms associated with EBP.…”

“… 1 , 2 Dystrophic nail changes may also be present as the first or only clinical sign of disease. 1 , 4 …”

Epidermolysis Bullosa Pruriginosa treated with baricitinib: A case report

“…Currently, EBP is treated with topical corticosteroids, tacrolimus, and oral thalidomide, but the outcomes are often unsatisfactory. [2] Baricitinib is a competitive inhibitor of the Janus Kinase (JAK) family of non-receptor protein kinases, predominantly acting against JAK-1 and JAK-2 subtypes. [3] Approved for severe cases of alopecia areata and moderate-severe atopic dermatitis in adults.…”